Cardiomyopathies & Cardiac Tumors Flashcards
What are the three types of cardiomyopathy?
Dilated
Hypertrophic
Restrictive
What are the causes of dilated cardiomyopathy?
Genetics, idiopathic, alcoholism (Happy holiday syndrome)
What type of cardiomyopathy can ‘Happy holiday’ syndrome cause?
Dilated cardiomyopathy
What are the clinical features of dilated cardiomyopathy?
- Myocardium becomes flabby, weak and pale
- compensatory ventricular hypertrophy & dilation
- Exercise intolerance progresses to congestive heart failure
- 75% die within 5 years of onset without transplant, due to ventricular arrythmias (50%)
What is hypertrophic cardiomyopathy?
- altered sarcomere function due to thick wall and small cavity of LV
- papillary mm and trabeculae carnae are prominent and encroach on lumen
- often asymmetric hypertrophy of interventricular septum
A 16 year old star soccer player collapses on the field in the fourth quarter and expires from a massive cardiac failure of his left ventricle. What cardiomyopathy does this pattern indicate?
Hypertrophic cardiomyopathy
A hypertrophic cardiomyopathy is an example of what type of aortic stenosis?
Subvalvular
What is restrictive cardiomyopathy?
A group of diseases in which myocardial or endocardial abnormalities limit diastolic filling while contractile function is normal
What pathologies can cause restrictive cardiomyopathy?
- Systemic amyloidosis
- hemachromatosis (^iron) (genetic & storage diseases)
What type of cardiomyopathy can be caused by hemachromatosis and systemic amyloidosis?
Restrictive cardiomyopathy
When does a myxoma present in life?
Adulthood
What is the cell of origin for a myxoma?
Connective tissue (fibroblast)
What is the preferential location for a myxoma?
Left atrium
49 year old patient presents with a heart tumor in his left atrium composed of fibroblasts. What is the name given to this gelatinous mass?
Myxoma
What age does a rhabdomyoma of the heart onset?
Pediatric
What is the most common pediatric heart tumor?
Rhabdomyoma
What is the cell of origin for a rhabdomyoma?
Striated cardiac myocytes
Where does a rhabdomyoma preferentially exist?
Walls of ventricles
8 year old male patient presents with a tumor in the right ventricle of his heart composed of striated cardiac myocytes. What type of tumor is described?
Rhabdomyoma
What is a hamartoma?
benign tumor composed of the primary tissue of an organ
What cardiomyopathy can be caused by holiday heart syndrome or Saturday night syndrome? Why?
Dilative cardiomyopathy due to a B1 deficiency
In a restrictive cardiomyopathy, the heart has trouble in (Systole/Diastole)?
Diastole
What is the most common etiology of sudden cardiac death in people under 35 years of age?
Hypertrophic cardiomyopathy
What is the most common etiology of sudden cardiac death in people over 35 years of age?
Coronary heart disease
What is the most common cardiac primary tumor in adults?
Myxoma
36 year old female patient is diagnosed with a mass in her heart. The mass is gelatinous upon biopsy measuring 5 cm. The mass appears to be a mix of connective tissue and is situated in the left atrium. What is the diagnosis?
Myxoma
A rhabdomyoma is a (Benign/Malignant) neoplasm of (Smooth/Striated) muscle cells?
Benign, striated
9 year old male patient is diagnosed with a mass in the left ventricle of their heart. The mass appears to be benign. What is the most common tumor based on this patients age and location?
Rhabdomyoma
Metastasis to the heart is common (TRUE/FALSE)?
FALSE
What cancers most commonly metastasize to the heart?
Lung, breast, and GI tract
How does dilated cardiomyopathy result in reduced stroke volume and cardiac output?
poorly contracting heart (weak) causes ^stretch & compliance -> valves become regurgitant -> less blood pumped out
What is dilated cardiomyopathy?
Genetic defects in cytoskeletal proteins lead to hypothesis that defects in force transmission lead to development of a dilated, poorly contracting heart
What is the most notable histologic feature of hypertrophic cardiomyopathy?
myofiber disarray
What population is hypertrophic cardiomyopathy seen in?
young athletes, but can occur at any age
What are the symptoms of hypertrophic cardiomyopathy?
- asymptomatic, although at risk for sudden death
- angina, dyspnea, syncope which may progress to heart failure
What is the result of restrictive cardiomyopathy on blood flow?
decreased compliance -> decreased filling (diastole) -> decreased SV & Q
